Headache Medicine 2021, 12(3):264-267 p-ISSN 2178-7468, e-ISSN 2763-6178

264

ASAA

DOI: 10.48208/HeadacheMed.2021.39

Headache Medicine

Case Report

Red Ear Syndrome and migraine:

case report and review of this peculiar association

Juliana Baleki Borri

, Hilton Mariano da Silva Junior

1,2

Pontifícia Universidade Católica de Campinas, Campinas, São Paulo, Brazil;

Hospital Municipal de Campinas Doutor Mário Gatti, Campinas, São Paulo, Brazil

Abstract

Introduction

The "Red Ear Syndrome" (RES) is a rare condition (about 100 cases were published in 25

years). It is characterized by episodes of hyperemia of the ear associated with burning pain.

Although the association of this syndrome and primary headaches is contemplated in literature,

its etiology and treatments are still poorly dened.

The aim of this paper is to report a case of RES related to migraine and possible pathophy-

siologic mechanisms.

Case Report

A 31-year-old woman presented with stabbing pain and marked erythema and edema of

the right ear accompanied by burning and local hyperhidrosis. These attacks lasted 2 hours

on average, and either occurred spontaneously or were associated with migraine without

aura. The only means of relief during the attacks was cooling the local with ice. Extensive

laboratory-chemical, microbiological, ear-nose-throat, clinical and neurological examinations,

magnetic resonance of the brain and cervical spine were unremarkable.

Conclusion

Uncertainty about the etiology of this syndrome is an obstacle to treatment. The frequent re-

lationship between RES and migraine suggests that is necessary to investigate the syndrome

in migraineurs. Furthermore, new reports about this disorder are important to increase the

knowledge of physicians, to reduce the delay in diagnosis and suffering of patients.

Juliana Baleki Borri

juliana.baleki@gmail.com

Edited by:

Marcelo Moraes Valença

Keywords:

Migraine

migraine disorders

Erythema

Pain

Red ear syndrome

Received: November 29, 2021

Accepted: December 6, 2021

265

ASAA

Borri JB, Silva Junior HMS

Red Ear Syndrome and migraine: case report and review of this peculiar association

Introduction

he "Red Ear Syndrome" (RES) is a rare condition. Its

etiology and treatments are still poorly dened. It is char-

acterized by episodes of hyperemia of the ear associated

with burning pain. RES can be associated with primary

headaches. This relationship was rst described in 1996,

when Lance published a series of cases on the syndrome,

and two patients had both RES and migraine.

From this,

other reports emerged and one of the hypotheses is that the

red ear phenomenon shares pathophysiological mechanisms

with migraine.

This syndrome can be classied into two forms. The

idiopathic form is often associated with primary headaches,

such as migraine and trigeminal autonomic headache.

Nevertheless, it may occur spontaneously and is more

frequent in young people. The secondary form, on the

other hand, is related to structural disorders, such as lesions

and irritations of C3 and C4 roots or temporomandibular

dysfunction and are more frequent in older patients.

2,3

Case Report

We report a 31-year-old female patient, who had been

presenting for 2 years with acute pain, edema and

accentuated erythema in the right ear pavilion (Figure

1), occasionally accompanied by hyperhidrosis, and

local burning. These attacks lasted 2 hours on average,

they occurred either spontaneously or were induced by

emotional stress or direct contact with heat. All crises were

associated with hyperemia and redness of the ear, which

started a few minutes after the pain, and normalized with

its resolution. During attacks, cooling was the only means

of relief.

Figure 1. Edema and accentuated erythema in the right ear pavilion.

The patient has also complained of migraine without

aura since adolescence, and reported a clear association

between the syndrome and some migraine attacks. The

patient used unarizine for both headache and red ear,

but treatment was interrupted due to side effects.

Extensive laboratory-chemical, microbiological, ear-nose-

throat, clinical and neurological examinations, as well as

magnetic resonance of the brain and cervical spine were

unremarkable.

Discussion

Epidemiology

According to the description of signs and symptoms,

associated to normal exams and absence of structural

lesions, the patient ts into the classication of primary

RES. This syndrome appears to be more prevalent in

women, in the adult population, and is more prevalent

than imagined in the pediatric population, mostly male.

4-8

The median age of RES onset is 44 years old, ranging from

4 to 92 years.

Due to the scarcity of case reports, prevalence and

incidence of RES are unknown. Notwithstanding, Raieli et

al.

, in a study with 92 participants aged 6 to 18 years,

reported that 24% of migraneurs patients had RES. That is a

relatively high number for such an unknown phenomenon.

This suggests that the syndrome should be investigated in

migraine patients. Furthermore, RES may be less rare than

expected, especially occurring during primary headache,

266

ASAA

Borri JB, Silva Junior HMS

Red Ear Syndrome and migraine: case report and review of this peculiar association

and considered as a symptom.

Clinical presentation

Episodes may occur concomitantly with primary headache

attacks, more common with migraine, or occur isolated in

patients with both conditions. In children, the occurrence

of red ear during migraine episodes is more common.

However, the frequency of red ear associated with migraine

is rare, it does not occur in all headache episodes.

The

frequency of attacks also varies and is similar to the

frequency of migraine in migraine patients.

4,8

The attacks

may occur with a frequency ranging from several a day to

a few per year.

Duration may vary from minutes to hours,

most lasting between 30 minutes to 1 hour.

5,6

In idiopathic cases, the main triggering factors for attacks

are temperature changes and tactile stimuli, as touch or

friction, mostly. Nevertheless, episodes could also occur

spontaneously.

Attacks may be bilateral, unilateral and

alternate sides. Ipsilaterality and duration are signicant

implications for the understanding that RES is directly

related with migraine. When red ear occurs alone, it has

been proposed that it may be an episode of an “acephalgic

migraine”.

The occurrence of bilateral attacks of red ear is

more common in children with migraine.

In most cases, pain accompanied by ear erythema are

the only symptoms. Other autonomic phenomena may be

present during the attacks, as in the case presented in this

investigation. The most common autonomic phenomena

are hyperhidrosis, edema and tearing. Such phenomena,

added to the short duration of episodes, suggest that RES

may be a form of Trigeminal Autonomic Cephalalgia.

9,10

Notwithstanding, the association between RES and

migraine is more common in literature than TAC´s.

2,4,6

review of literature including about 60 cases, showed that

55% of patients with primary RES suffer from migraine.

Physiopathology

According to the proposed pathophysiological hypothesis

for the frequent association between migraine and RES, it

is plausible that both conditions share pathophysiological

mechanisms. It has been proposed that, during migraine

attacks, a trigemino vascular activation results in

vasodilation by direct release of vasodilator peptides such

as substance P, CGRP and others.

This activation would

explain the pain that may extend beyond the trigeminal

innervation territory due to the overlap between trigeminal

and upper cervical spinal nerves in the trigemino-cervical

complex. This anatomical relationship may explain the

occurrence of neck pain in patients with migraine.

Another mechanism that might account is the sensory

innervation promoted by the trigeminal nerve in the external

carotid artery. Ergo, an activation in its nucleus could

trigger an antidromic impulse and release of vasodilating

substances in this vessel responsible for the blood supply

to the ear.

In addition, some migraine episodes may result

in facial pallor due to an imbalance between sympathetic

and parasympathetic vasomotor innervation.

When the red ear phenomenon appears during migraine,

it is possible to observe facial vasoconstriction and ear

vasodilation, indicating the activation of different neuronal

modules.

The same authors showed that RES preceding

migraine attack, such as an aura phenomenon, may

indicate the syndrome has an isolated neuronal system

which can be activated during the migraine.

On the other hand, when RES occurs after migraine,

some authors believe that it is an atypical migraine with

characteristics of migraine and other primary headaches,

and they justify it by the modular theory.

This theory

proposes that a group of neurons called modules are

anatomically linked to one another and become activated

in a manner characteristic for the individual. The theory

could also explain why not all migraine attacks are

accompanied by RES (before, during or after) even in

patients suffering from both conditions.

Even when the

attacks are not time related, the history of migraine should

be investigated. Raieli et al.

showed that 50% of isolated

RES cases have also a history of migraine.

The association between RES and migraine

When both conditions are related, the red ear could be

considered a sign of hyper parasympathetic activation via

trigeminal-autonomic reex during migraine, which leads

to consider RES a phenomenon related to migraine.

This

activation is also consistent with the phenomenon of red

ear in primary headaches with autonomic involvement and

could explain the existence of autonomic features in patients

with migraine.

Furthermore, mechanisms in migraine are

not only capable of stimulating the trigemino-autonomic

reex, but can also stimulate a cervical autonomic reex

resulting in the RES.

In addition to the occurrence of both disorders in the same

patient, what supports the hypothesis that RES shares

pathophysiological mechanisms in common with primary

headaches is the resolution or a reduction in frequency

and severity of RES. It occurs when treatment is initiated for

the coexisting migraine. Beta-blocker and calcium channel

blocker (unarizine and nimodipine) showed a good

response in both disorders.

3,9

However, because there

are many potential causes, there is not a single treatment

suggested, and many patients are treatment resistant.

267

ASAA

Borri JB, Silva Junior HMS

Red Ear Syndrome and migraine: case report and review of this peculiar association

The importance to know the pathophysiology is to

identify a therapeutic target and treatment. Several drugs

for headache and pain were used in patients with this

syndrome. Nonetheless, it is considered refractory to

several drugs and varies from person to person.

More studies with the adult population are needed to

identify the prevalence of RES. The relationship between

RES and primary headaches, mainly migraine, is strongly

suggested. Thus, RES should be investigated in all patients

with migraine.

Author contributions: The authors contributed equally in

the preparation of the manuscript.

Conflict of interest: There is no conict of interest.

Funding: The authors received no nancial support for the

research, authorship, and/or publication of this article.

Juliana Baleki Borri

https://orcid.org/0000-0003-2590-6426

Hilton Mariano da Silva Junior

https://orcid.org/0000-0002-9778-9946

References

1. Lance JW. The red ear syndrome.

Neurology

1996;47(3):617-620 Doi:10.1212/wnl.47.3.617

2. Donnet A and Valade D. The red ear syndrome.

J Neurol Neurosurg Psychiatry

2004;75(7):1077

Doi:10.1136/jnnp.2003.030742

3. Purdy RA and Dodick DW. Red ear syndrome.

Curr Pain

Headache Rep

2007;11(4):313-316 Doi:10.1007/

s11916-007-0210-8

4. Raieli V, Monastero R, Santangelo G, Eliseo GL, Eliseo

M and Camarda R. Red ear syndrome and migraine:

report of eight cases.

Headache

2002;42(2):147-151

Doi:10.1046/j.1526-4610.2002.02033.x

5. Raieli V, Pandol E, La Vecchia M, Puma D, Calò

A, Celauro A and Ragusa D. The prevalence of

allodynia, osmophobia and red ear syndrome in the

juvenile headache: preliminary data.

J Headache

Pain

2005;6(4):271-273 Doi:10.1007/s10194-005-

0205-y

6. Raieli V, Compagno A, Brighina F, La Franca G,

Puma D, Ragusa D, . . . D'Amelio M. Prevalence

of red ear syndrome in juvenile primary

headaches.

Cephalalgia

2011;31(5):597-602

Doi:10.1177/0333102410388437

7. Ryan S, Wakerley BR and Davies P. Red ear

syndrome: a review of all published cases (1996-

2010).

Cephalalgia

2013;33(3):190-201

Doi:10.1177/0333102412468673

8. Raieli V, Compagno A and D'Amelio M. Red Ear

Syndrome.

Curr Pain Headache Rep

2016;20(3):19

Doi:10.1007/s11916-016-0547-y

9. Lambru G, Miller S and Matharu MS. The red

ear syndrome.

J Headache Pain

2013;14(1):83

Doi:10.1186/1129-2377-14-83

10. Al-Din AS, Mir R, Davey R, Lily O and Ghaus N.

Trigeminal cephalgias and facial pain syndromes

associated with autonomic dysfunction.

Cephalalgia

2005;25(8):605-611 Doi:10.1111/j.1468-

2982.2005.00935.x

11. Marinis M and Accornero N. Recurrent neck pain

as a variant of migraine: description of four cases.

Neurol Neurosurg Psychiatry

1997;62(6):669-670

Doi:10.1136/jnnp.62.6.669

12. Young WB, Peres MF and Rozen TD. Modular

headache theory.

Cephalalgia

2001;21(8):842-849

Doi:10.1046/j.1468-2982.2001.218254.x